Idiopathic Pulmonary Fibrosis (IPF) is an idiopathic interstitial pneumonia characterized by the rapid replacement of the lung parenchyma with fibrosis which results in progressive deterioration of lung function. This process often leads to respiratory failure and death. Although several drugs are under investigation, currently there are no effective means of treating this devastating disease. This proposal is submitted in response to the NIH-sponsored RFA titled "IPF Clinical Research Network" with the intention of becoming one of the clinical research centers within this network. Our site will be based at the Emory Center for the Treatment and Study of Interstitial Lung Disease (The Emory ILD Center). The Emory ILD Center is considered a premier center for the diagnosis, treatment, and research of patients with IPF and related disorders because: 1) it currently serves as a referral base for patients with interstitial lung disease identified at any of five Emory hospitals, at several academic institutions in metro Atlanta, and through a network of private practice physicians located throughout the state of Georgia and the Southeastern region of the country;2) it has served as a site for clinical-based investigations related to IPF and other interstitial lung diseases, and as such has developed extensive experience in clinical trials and translational research related to IPF;3) it is staffed by a multidisciplinary team of clinical investigators, translational researchers, research coordinators, and other staff with expertise in the diagnosis and management of patients with IPF;and 4) it is directed by a team of investigators recognized in the field for their work in elucidating the mechanisms of lung tissue remodeling and fibroproliferation. The proposal describes two clinical trials to be considered by the Network that target pro-fibrotic pathways and the lung fibroblast/myofibroblast, the key effector cell responsible for fibroproliferation and tissue remodeling in pulmonary fibrosis. It also includes a Clinical Research Skills Core that will mentor junior investigators into developing the necessary skills to become independent clinician scientists involved in research related to IPF. The team of investigators leading the clinical research center and the Clinical Research Skills Core are currently engaged in clinical research related to IPF and have developed a reputation for mentoring young investigators into careers in academia. (End of Abstract)